34例非阑尾来源腹膜假黏液瘤诊治分析

  目的  探讨非阑尾来源腹膜假黏液瘤（pseudomyxoma peritonei,PMP）的临床病理特征和诊疗经验。  方法  回顾性分析2011年9月至2019年2月于航天中心医院采用肿瘤细胞减灭术（cytoreduction surgery,CRS）联合腹腔热灌注化疗（hyperthermic inter-peritoneal chemotherapy,HIPEC）治疗的34例非阑尾来源PMP患者的临床资料,总结其临床表现及影像学特征,同时利用Log-rank检验对相关因素进行生存分析。  结果  本研究34例患者临床表现主要为腹胀（58.8%）和腹盆腔包块（52.9%）;CRS+HIPEC治疗后主要并发症发生率为14.7%,在随访中9例患者死亡,1、3年生存率分别为69.6%、53.5%;单因素分析中,腹膜癌指数（peritonealcancer index,PCI）>20、未行灌注化疗以及非根治性手术是预后不良的显著危险因素,而性别、年龄、组织来源、病理类型等未体现出显著相关性。  结论  非阑尾来源PMP无特异性临床表现,术前较难判断原发病灶,确诊需要依靠术后病理及免疫组织化学检测。但无论来源如何,均以腹腔内广泛肿瘤种植和局部浸润为主要临床表现,CRS+HIPEC是安全有效的治疗手段。      

细胞减灭术+腹腔热灌注化疗治疗腹膜假黏液瘤——单中心854例经验总结

目的:腹膜假黏液瘤(pseudomyxoma peritonei,PMP)是一种罕见的临床综合征,细胞减灭术(cytoreductive surgery,CRS)联合腹腔热灌注化疗(hyperthermic intraperitoneal chemotherapy,HIPEC)因其良好的治疗效果已经成为各中心公认的标准治疗方案,航天中心医院从2008年开始致力于PMP患者诊治工作,2016年开始将全腹膜切除应用于临床,本研究通过对既往资料进行收集整理,对PMP治疗经验进行总结。方法:回顾分析2008年1月到2019年1月航天中心医院腹膜假黏液瘤中心收治并接受手术治疗的PMP患者临床资料及随访结果。对所有患者的手术方式、根治程度及并发症发生情况等临床资料进行搜集整理,并通过随访结果对相关因素进行生存分析,了解CRS+HIPEC在PMP治疗中的临床价值,同时对腹膜切除技术治疗效果进行评价。结果:共纳入854例患者,平均年龄50岁,中位改良腹膜肿瘤指数(peritoneal cancer index,PCI)为29,其中25.5%的患者接受了根治性手术切除,细胞减灭程度(completene...     

肿瘤细胞减灭术加腹腔热灌注化疗治疗腹膜假黏液瘤182例分析

  目的  腹膜假黏液瘤（pseudomyxoma peritonei,PMP）是一种主要来源于阑尾黏液性肿瘤的恶性肿瘤综合征,肿瘤细胞减灭术（cytoreductive surgery,CRS）加腹腔热灌注化疗（hyperthermic intraperitoneal chemotherapy,HIPEC）是国际推荐的PMP标准治疗。本研究旨在评估CRS+HIPEC治疗PMP的疗效及围手术期安全性。  方法  研究首都医科大学附属北京世纪坛医院2001年1月至2008年5月采用CRS+HIPEC治疗182例PMP临床数据库,进行生存分析,通过单因素和多因素分析筛选独立预后因素,并分析围手术期安全性。  结果  182例PMP患者接受CRS+HIPEC治疗,低级别PMP 73例（40.1%）,部分低级别、部分高级别PMP 50例（27.5%）,高级别PMP 53例（29.1%）,PMP伴印戒细胞6例（3.3%）;中位腹膜癌指数（peritoneal cancer index,PCI）30分,PCI≥20分为134例（74.0%）;肿瘤细胞减灭程度（completeness of cytoreduction,CC）评分0~1分者为79例（44.1%）;死亡48例（26.4%）,生存134例（73.6%）,中位生存时间64.7个月（95%CI:43.1~84.3个月）。Cox多因素回归分析发现4个独立预后因素:年龄（HR=12.079,95%CI:1.605~90.916）、CC（HR=0.211,95%CI:0.069~0.641）、是否有吻合口（0个vs. >1个）（HR=5.519,95%CI:1.176~25.907）、吻合口数量（1个vs. >1个）（HR=7.543,95%CI:1.592~35.732）。围手术期死亡率、严重不良事件率分别为1.6%、19.8%。  结论  PMP患者在腹膜肿瘤专科单位接受CRS+HIPEC治疗,达到完全肿瘤细胞减灭,可延长生存,围手术期安全性可接受。      

腹膜假黏液瘤的诊断与治疗研究进展

摘 要：腹膜假黏液瘤是一种罕见的临床综合征,主要来源于阑尾黏液肿瘤,以腹腔内充满黏液或胶冻样腹水为特征。发病率每年约3/100万～4/100万。由于其进展缓慢,无特异性临床症状,容易导致误诊。超声、CT等影像学检查有助于诊断。目前细胞减灭术与腹腔热灌注化疗相结合的综合治疗策略已经成为腹膜假黏液瘤标准的治疗方法,然而术后高复发率仍是亟待解决的难题。肿瘤减灭程度是影响生存预后的重要因素,而全身化疗能否获益尚存在争议。     

雷替曲塞用于腹膜假黏液瘤肿瘤细胞减灭术后腹腔热灌注化疗的短期疗效及安全性分析

目的:观察雷替曲塞用于腹膜假黏液瘤(pseudomyxoma peritonei,PMP）肿瘤细胞减灭术(cytoreductive surgery,CRS)后腹腔热灌注化疗(hyperthermic intraperitoneal chemotherapy,HIPEC）的短期疗效及安全性。方法:回顾性分析我院自2019年01月至2020年03月接受CRS联合HIPEC治疗的PMP患者,根据术后灌注药物方案是否应用雷替曲塞分为观察组和对照组。观察组术后第1次灌注药物为雷替曲塞(4 mg),第2~5次灌注药物为5-氟尿嘧啶（5-FU）(1 g);对照组术后5次灌注药物均采用5-FU(1 g)。比较两组患者术后一般情况、手术并发症发生率、排气时间、住院时间及两组患者手术前后血常规(WBC、PLT)、肝功能(ALT、AST)、肾功能(Cr)变化情况。结果:研究期间接受CRS及HIPEC治疗的患者共86例,其中观察组和对照组分别为39例和47例。两组基线资料一致(P＞0.05)。两组间术后出血、肠瘘、胸腔积液、切口感染等并发症发生率比较无统计学差异（P＞0.05）。化疗相关不良反应显示对照组腹痛发生率较观察组多(P=0.044),其余不良反应如恶心、呕吐、乏力、发热3日以上等两组比较均无统计学差异（P＞0.05）。两组患者手术前后血液学检查结果变化无统计学差异（P＞0.05）。结论:雷替曲塞用于PMP CRS后HIPEC,不增加术后并发症,不良反应可耐受,治疗过程安全性良好。     

肿瘤细胞减灭术联合腹腔热灌注化疗治疗腹膜假黏液瘤的围手术期安全性研究

目的探讨肿瘤细胞减灭术(CRS)联合腹腔热灌注化疗(HIPEC)治疗腹膜假黏液瘤(PMP)的围手术期安全性, 分析严重不良事件(SAEs)的影响因素。方法回顾性分析254例PMP患者CRS+HIPEC围手术期SAEs的发生情况, 通过单因素和多因素分析筛选CRS+HIPEC围手术期SAEs的独立危险因素。结果 254例PMP患者共接受272例次CRS+HIPEC, 共发生围手术期SAEs 93例次(34.2%), 其中感染26例次, 消化系统22例次, 呼吸系统17例次, 心血管系统15例次, 血液系统8例次, 泌尿系统4例次;Ⅲ级76例次, Ⅳ级13例次, Ⅴ级4例次。单因素分析显示, HIPEC药物方案(P=0.020)、术中红细胞输注量(P=0.004)、术中出血量(P=0.002)与SAEs的发生有关。多因素分析显示, 术中红细胞输注量是围手术期SAEs的独立危险因素(OR=1.160,P=0.001)。结论 CRS+HIPEC治疗PMP的围手术期安全性可接受, 应注意减少术中出血、红细胞输注量, 预防SAEs的发生。     

基于真实世界数据的腹膜假黏液瘤诊治现状分析

  目的  基于真实世界数据分析腹膜假黏液瘤患者的诊治现状、自然病程及预后因素。  方法  回顾性分析2009年2月至2020年7月在首都医科大学附属北京世纪坛医院就诊的具有完整自然病程的腹膜假黏液瘤患者的相关资料,包括临床病理特征、非规范化治疗情况（误诊时间、误治时间、既往抗肿瘤治疗情况）、肿瘤细胞减灭术+腹腔热灌注化疗（cytoreductive surgery+hyperthermic intraperitoneal chemotherapy,CRS+HIPEC）治疗情况[手术时间、术中输血情况、腹膜癌指数（peritoneal cancer index,PCI）评分、细胞减灭程度（completeness of cytoreduction,CC）评分、脏器切除数量、腹膜切除区域数量、严重不良事件（serious adverse event,SAE）等]、随访生存时间,随访终点为患者死亡。采用Kaplan -Meier法绘制生存曲线,组间比较采用Log-rank检验。影响5年生存的预后因素采用Cox比例风险回归模型进行单因素和多因素分析。  结果  共纳入94例患者,其中男性57例（60.6%）,女性37例（39.4%）, 中位年龄54（24～76）岁,既往抗肿瘤治疗者59例（62.8%）,中位误诊时间0.8（0～62.5）个月,中位误治时间15.3（0～214.8）个月。所有患者均行CRS+HIPEC治疗,中位手术时间10.1（4.8～16.5）h,中位脏器切除数2（0～8）个,中位腹膜切除区域数5（0～9）个,中位PCI评分32（3～39）分,CC评分2～3分者达80.9%（76/94）,SAE发生率35.1%（33/94）。94例患者中位总生存期30.8（2.4～218.4）个月,1、2、3、5年生存率分别为96.8%、63.8%、44.7%和23.4%。分层分析显示,既往腹腔化疗（46.5 个月vs. 26.3个月）、PSS 1～3分（39.0个月 vs. 21.9个月）、低/高级别病理类型（41.5/40.9 个月vs. 20.1个月）、KPS≥80分（41.5 个月vs. 23.9个月）、无淋巴结转移（35.5 个月vs. 17.1个月）、Ki-67<50%（46.4 个月vs. 20.8个月）的患者中位生存时间延长（P<0.05）。5年生存预后分析中,单因素分析显示以下5个因素与5年生存率有关:PSS评分（P=0.021）、既往腹腔化疗（P=0.008）、病理类型（P=0.004）、淋巴结转移（P=0.008）和Ki-67表达程度（P=0.003）。多因素分析显示出以下3个影响5年生存的独立预后因素:既往腹腔化疗（HR=0.458,95%CI:0.253～0.827,P=0.010）、淋巴结转移（HR=2.879,95%CI:1.345～6.163,P=0.006）、Ki-67≥50%（HR=2.502,95%CI:1.418～4.417,P=0.002）。  结论  PMP非规范化治疗现象较普遍,误治时间长,淋巴结转移及Ki-67高表达是独立不良预后因素,CRS+HIPEC术前腹腔灌注化疗可能为PMP的治疗提供新的方向。      

细胞减灭术加腹腔热灌注化疗治疗腹膜种植瘤的研究进展

腹盆腔肿瘤局部进展所导致的腹膜种植瘤是致命性病变,传统治疗方法的效果非常有限,国际上近年来把细胞减灭术加腹腔热灌注化疗作为新治疗模式,作者分析这方面的研究进展。文献检索分析腹膜种植瘤的发病率,病理生理学基础,药物治疗学基础,介绍细胞减灭术加腹腔热灌注化疗治疗各类腹膜种植瘤的临床试验结果。胃癌、结直肠癌、卵巢癌、腹膜间皮瘤、腹膜假黏液瘤、腹腔肉瘤都可形成腹膜种植瘤,常规化疗的中位生存期约6个月。术中腹腔内热灌注化疗可使腹腔内药物浓度比血液内高10～1000倍,温热与化疗有协同抗肿瘤作用。腹膜种植瘤指数是判断癌症腹膜播散程度的客观标准,最大程度细胞减灭术加腹腔内热灌注化疗能显著延长病人的中位生存期:结直肠癌可达到20个月,胃癌达10个月,卵巢癌达65个月,围手术期病残率约27～56%、死亡率0～11%。大多数为Ⅱ期临床研究,Ⅲ期随机分组临床试验较少。细胞减灭术加腹腔热灌注化疗是目前治疗腹膜种植瘤的最有效方法,需要前瞻性多中心随机分组临床试验,以进一步优化技术,提高疗效。     

深部热疗联合5-Fu及顺铂腹腔化疗辅助治疗腹膜假黏液瘤的安全性及初步有效性研究

目的:研究深部热疗联合5-Fu及顺铂(DDP)腹腔化疗辅助治疗腹膜假黏液瘤(pseudomyxoma peritonei,PMP)的安全性和初步有效性.方法:回顾性分析2018年1月至2019年12月航天中心医院接受深部热疗联合腹腔化疗患者的临床资料及随访结果,分析血常规、肝肾功、Karnofsky评分及并发症发生情况...     

The Role of Microorganisms in Appendiceal Pseudomyxoma Peritonei: A Review

Pseudomyxoma peritonei (PMP) is a rare clinical syndrome. It originates from neoplasms of the appendix and leads to the formation of peritoneal implants and the accumulation of mucinous ascites. PMP represents a spectrum of low to high-grade disease. Despite aggressive management, many PMP patients recur, leading to debilitating symptoms and few treatment options. Therefore, scientists have continued to look for ways to improve treatment and further understand disease pathogenesis. Microorganisms were previously hypothesized to play a role in PMP progression and development. Hence, antibacterial treatment was suggested by some authors, but the data were limited. In this paper, we review the current data on the role of bacteria in PMP, discuss the significance, and suggest possible solutions to the inherent challenges in these studies. Given the limitations of the discussed studies, we remain skeptical about introducing novel antibacterial treatment into clinical practice at this time; however, the available data are valuable and indicate that more research into the molecular mechanisms of PMP is needed.     

Review: Pathology and Its Clinical Relevance of Mucinous Appendiceal Neoplasms and Pseudomyxoma Peritonei

Until recently, many classifications existed for the terminology and histopathologic classification of appendiceal mucinous neoplasms, mucinous appendiceal adenocarcinomas, and pseudomyxoma peritonei (PMP). A major accomplishment was achieved by consensus-based histopathologic classifications on behalf of the Peritoneal Surface Oncology Group International regarding mucinous appendiceal tumours and PMP. As different classifications were used over the years and also owing to the rare nature of these tumors, many clinicians are not familiar with the terminology and the impact on patient management. Hence, an overview concerning mucinous appendiceal neoplasms, mucinous appendiceal adenocarcinomas, and PMP is provided to serve as an introduction into the basic morphology of these tumors with tentative recommendations for management.     

Pseudomyxoma peritonei: natural history and treatment

Abstract

Pseudomyxoma peritonei (PMP) is an uncommon disease characterised by mucinous ascites, classically originating from a ruptured low grade mucinous neoplasm of the appendix. The natural history of PMP revolves around the “redistribution phenomenon”, whereby mucinous tumour cells accumulate at specific sites with relative sparing of the motile small bowel and to a lesser extent other parts of the gastrointestinal tract. Peritoneal tumour accumulates due to gravity and at the sites of peritoneal fluid absorption, namely, the greater and lesser omentum and the under-surface of the diaphragm, particularly on the right. The optimal treatment is complete macroscopic tumour excision termed cytoreductive surgery (CRS) combined with Hyperthermic Intra-Peritoneal Chemotherapy (HIPEC). Total operating time for complete CRS and HIPEC for extensive PMP is around 10?h and generally involves bilateral parietal and diaphragmatic peritonectomies, right hemicolectomy, radical greater omentectomy with splenectomy, cholecystectomy and liver capsulectomy, a pelvic peritonectomy with, or without, rectosigmoid resection and bilateral salpingo-oophorectomy with hysterectomy in females. A unique feature of low grade PMP, which differs from other peritoneal malignancies, includes slow disease progression, which may be asymptomatic until advanced stages. Additionally, very extensive disease with a high “PCI” (Peritoneal Carcinomatosis Index) may still be amenable to complete excision and cure. In cases where complete tumour removal is not feasible, maximum tumour debulking can still result in long-term survival in PMP. PMP is challenging, complex but nevertheless the most rewarding peritoneal malignancy amenable to cure by CRS and HIPEC.     

The Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in Ovarian Cancer

Overall outcomes for women with epithelial ovarian cancer (EOC) remain relatively poor, and superior methods of treatment are needed. EOC is a peritoneal surface malignancy that is relatively sensitive to chemotherapy agents, making it a good target for i.p. chemotherapy. Because there is strong laboratory data demonstrating the ability of hyperthermia to increase the efficacy of chemotherapeutic agents, the addition of hyperthermia to i.p. chemotherapy, hyperthermic intraperitoneal chemotherapy (HIPEC), makes theoretical sense. This article reviews the current literature and discusses the possible role for HIPEC in EOC at significant natural history time points: front line, at the time of interval debulking, in consolidation, and for recurrent disease. The conclusion is that much further research is needed but that HIPEC could sensibly be researched at all the natural history time points in EOC.     

The Impact of Computed Tomography Measurements of Sarcopenia on Postoperative and Oncologic Outcomes in Patients Undergoing Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy

Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) is a treatment option for peritoneal metastases (PM) but is associated with significant postoperative morbidity. The aim of this study was to determine the prognostic value of computed tomographic (CT)-measured sarcopenia on postoperative outcomes and survival in patients undergoing CRS-HIPEC for PM from various origins. A retrospective cohort study was conducted between 2012 and 2020. Three-hundred and twelve patients (mean age 57.6 ± 10.3, 34.3% male) were included, of which 88 (28.2%) were sarcopenic. PM from a colorectal origin was the most common in both groups. The proportion of major postoperative complications (Clavien-Dindo ≥ III) was not higher in the sarcopenic group (15.9% in sarcopenic patients vs. 23.2% in nonsarcopenic patients, p = 0.17). The mean Comprehensive Complication Index scores, HIPEC-related toxicities, length of hospital stay, and duration of parenteral nutrition were comparable regardless of sarcopenia status. In the multivariate logistic regression analysis of severe complications, only peritoneal carcinomatosis index reached statistical significance (OR, 1.05; 95% CI, 1.01 to 1.08, p = 0.007). Sarcopenia did not impact origin-specific overall survival on Cox regression analysis. Sarcopenia was not associated with worse rates of postoperative severe complications or worse survival rates. Future prospective studies are required before considering sarcopenia as part of preoperative risk assessment.     

腹腔热灌注化疗在腹膜癌中的应用现状

腹膜癌是一种在腹膜上发生和发展的一类恶性肿瘤,包括原发性和继发性两种.腹腔热灌注化疗是一种结合腹腔灌注、热疗和化疗的腹膜癌综合治疗方法,能够得到较为满意的疗效.近年来,随着技术的不断进步,研究的不断深入,腹腔热灌注化疗技术已经成为治疗腹膜癌最有效的方法之一.本文就腹腔热灌注化疗技术在腹膜癌中应用现状展开综述.     

腹腔热灌注化疗在胃癌腹膜转移治疗中的作用

胃癌发生腹膜转移预后极差,中位生存期小于6个月.全身化疗并没有明显增加这些患者的生存期.腹腔热灌注化疗是目前常用的治疗腹膜转移的方法.全文系统回顾分析了腹腔热灌注化疗在胃癌腹膜转移治疗、进展期胃癌术后辅助治疗以及术前新辅助治疗的疗效.     

Validating the PSOGI classification of peritoneal disease from non-carcinoid epithelial appendiceal neoplasms in the curative and palliative setting: an observational retrospective study

BackgroundFew studies on long-term survival have been published since the new updated pseudomyxoma peritonei (PMP) classification was published in 2016. The aim was to investigate long-term survival according to the Peritoneal Surface Oncology Group International (PSOGI) classification and compare prognostic factors.MethodsFrom Uppsala University Hospital, consecutive patients referred for cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) from 2004 to 2017 with peritoneal disease from non-carcinoid mucinous epithelial appendiceal neoplasms were included in the study. The peritoneal disease was divided into four groups: mucin only, low-grade mucinous carcinoma peritonei (MCP-1), high-grade (MCP-2), and high-grade with signet ring cells (MCP-3). Survival curves were rendered, and prognostic factors were compared.ResultsThe study included 223 patients: 36 with mucin only, 112 with MCP-1, 70 with MCP-2, and 5 with MCP-3. Thirty-eight patients had a palliative debulking or open/close procedure. The 5- and 10-year overall survival was 97% and 97% for mucin only, 83% and 70% for MCP-1, 69% and 49% for MCP-2, with no patients still under follow-up after 5 years in the MCP-3 group. In a multivariable analysis, completeness of cytoreduction (CC) score 2–3 and PSOGI class MCP-3 were significantly associated with lower survival. The 5-year overall survival in the palliative setting was 40% vs. 44% (MCP-1 vs. MCP-2, P>0.05) with median survival 51 vs. 53 months, respectively.ConclusionsThe PSOGI classification of PMP provides a solid differentiation of prognostic groups after CRS/HIPEC treatment, but not in the palliative setting.     

高精度持续循环腹腔热灌注化疗联合静脉化疗治疗卵巢癌的临床研究

目的：探讨高精度持续循环腹腔热灌注化疗（HIPEC）联合静脉化疗治疗卵巢癌的临床疗效。方法入组卵巢癌患者198例,分为2组,其中治疗组120例（HIPEC 联合紫杉醇＋奥沙利铂方案静脉化疗组）、对照组78例（紫杉醇＋奥沙利铂方案静脉化疗组）。观察2组患者的生存时间、病死率、复发率、生活质量及化疗毒副反应。结果治疗组患者术后1 a、2 a、3 a 病死率及复发率明显低于对照组,而平均生存时间高于对照组（P ＜005）。结论 HIPEC 联合静脉化疗能有效降低卵巢癌患者术后复发率及病死率,提高生活质量,延长其生存时间。     

洛铂联合多西他赛行肿瘤细胞减灭术加腹腔热灌注化疗治疗腹膜癌的临床观察

目的 分析洛铂联合多西他赛行肿瘤细胞减灭术（cytoreductive surgery, CRS）加腹腔热灌注化疗（hyperthermic intraperitoneal chemotherapy, HIPEC）治疗腹膜癌（peritoneal carcinoma, PC）的围手术期安全性及疗效。 方法 PC患者行CRS+HIPEC治疗,药物为洛铂50 mg/m2、多西他赛60 mg/m²,加入12 000 ml 0.9%氯化钠溶液加热至（43±0.5）℃持续灌注60 min。记录术后6天体温和心率变化、围手术期不良事件、血常规及血生化指标、术后患者恢复情况及生存结果。结果 90例PC患者行95次CRS+HIPEC,手术时间180~450 min (中位数485 min）;术后6天最高体温、心率分别为36.4℃~38.6℃（中位数37.5℃）、76~124 bpm（中位数100 bpm）,严重不良事件16例,包括围手术期死亡2例。中位生存期20.8月（95%CI: 13.1~25.8月）,1、3、5年生存率分别为75.6%、45.6%、43.3%。 结论 洛铂联合多西他赛进行CRS+HIPEC治疗PC安全性可接受,有助于延长患者生存期。